Transitional epithelial cancers in upper urinary tract are quite rare. It is less often being synchronous with the bladder cancer and this situation needs closer follow-up of the patient. In this case report, we evaluated a 56 years-old male having a giant hydronephrosis and nonfunctional kidney, urethra tumor in the light of current literature. The patient presented with right flank pain, palpable abdominal mass and intermittent hematuria in last one years.